Comparison of radiographic and clinical characteristics of low-risk and high-risk cystic fibrosis genotypes.

BACKGROUND: Patients with cystic fibrosis (CF) exhibit a wide range of disease severity, and can be broadly stratified into high-risk and low-risk groups based on cystic fibrosis transmembrane conductance regulator (CFTR) mutation class. Patients with a low-risk genotype are often diagnosed as adults, with milder disease and lower sweat chloride values. The aim of the current study was to better understand radiographic and clinical characteristics of sinus disease in adult CF patients within this risk category. METHODS: Adult CF patients were retrospectively compared to a control group of patients with chronic rhinosinusitis. CF diagnostic testing and pulmonary characteristics were compared between high-risk and low-risk CF groups, and sinus CT findings were compared among all 3 groups. RESULTS: When comparing CF cohorts (n = 25 and 30, respectively), earlier age at diagnosis (p < 0.001), higher sweat chloride values (p < 0.001), lower forced expiratory volume in 1 second (FEV1 ) values (p < 0.001), and a higher prevalence of pulmonary infection with Pseudomonas aeruginosa (p = 0.001) were found in the high-risk genotype group. A significantly increased incidence of sinus hypoplasia/aplasia and bony sclerosis was seen when comparing both CF groups to the control cohort (n = 30), as well as when comparing the high-risk and low-risk CF genotype cohorts. CONCLUSION: The current study describes clinicopathologic findings of sinus disease in adult CF patients in the context of genotype severity. Our data demonstrate that while patients within a low-risk genotype cohort have generally milder lung disease, they retain classic radiographic findings of CF sinus disease that can help raise the index of suspicion for undiagnosed CF.

Swing technique for middle turbinate preservation in expanded endonasal skull base approaches.

Endoscopic endonasal approaches to the middle fossa and orbital apex have traditionally included resection of the middle turbinate to improve visualization and operating space. The aim of this publication is to demonstrate a surgical technique that affords similar visualization and space but preserves the middle turbinate. We describe a technical modification that allows for conservation of the middle turbinate and describe an illustrative case. As current surgical techniques evolve towards progressively less morbidity, preservation of anatomic structures such as the middle turbinate will be pursued. In the case described, middle turbinate preservation did not negatively affect access or visualization and did not appear to alter postoperative wound healing. With middle turbinate preservation, the principle function of airflow conditioning and potential neural regeneration are maintained.